methylprednisolone pulse therapy for coronary artery dilatation or aneurysm formation in kawasaki disease

Conditions: Mucocutaneous Lymph Node Syndrome

Summary: in this study, the investigator plan to prescribe methylprednisolone pulse therapy in kawasaki disease patients with coronary artery lesions or aneurysm formation beyond acute stage to investigate the role for vasculitis of kd or regression of dilatation.

kawasaki disease is the most common systemic vasculitis in children. coronary artery aneurysms may develop in 20-25% of untreated patients. intravenous immune globulin (ivig) can reduce coronary-artery aneurysms to 3-5%. numerous studies and clinical trials had pointed out that corticosteroid treatment (pulse therapy or not) could lower the incidence of coronary artery abnormality in high-risk kd patients. however, the therapeutic effect of corticosteroid in kd patients with aneurysm formation after acute stage was never mentioned. there is no effective treatment for aneurysm formation available in kd after acute stage. methylprednisolone pulse therapy (mp pulse) was used for treatment of kd during acute stage since more than 20 years ago. mp pulse plus ivig seems not benefit for kd patients but benefit for ivig resistant kd patients or for high-risk group of cal formation/ ivig resistance group. mp pulse therapy is well document used in autoimmune disease vasculitis such as sle, rheumatoid arthritis, dermatomyosis...etc. taking together, mp pulse is effective and safe for kd patients during acute stage. in this study, the investigators plan to use mp pulse in kd patients with cal or aneurysm formation beyond acute stage to investigate the role of vasculitis of kd or regression of dilatation. methods: the investigators conducted a prospective study of methylprednisolone pulse therapy (mp pulse) for kd patients with coronary aneurysm or dilatation formation. the investigators will enroll these patients to receive methylprednisolone pulse (mp pulse, 30mg/kg, max:1g/day for continue 3 days) for treatment. together with other anti-inflammatory oral medicine including monteleukast, dextromethorphan(dxm), prednisolone, and ketotifen as supplementary treatment. the specific aim of this study is the regression of coronary artery aneurysm after mp pulse therapy. under the hypothesis and specific aim, the investigators plan to do in the following 3 years: 1. during the 1st year, the investigators will enroll for 5-10 cases for safety surveys including blood pressure monitoring, inflammatory markers, liver function, renal function, electrolyte imbalance, growth problems as phase i study. 2. in the 2nd and 3rd year of this study, the investigators will enroll for 20-30 cases for an effective survey as phase ii study. results from this study will help clinicians to treat aneurysm formation or coronary artery dilatation in kd patients and reduce the activity limitation of patients, reduce the medical resource in those patients. the investigators may provide the first treatment for aneurysm in kd.

Eligibility criteria:
inclusion criteria: * kawasaki disease patients with coronary artery dilation or aneurysm formation after acute stage (at lease 3 weeks after ivig treatment) exclusion criteria: * patients meet the contraindications of methylprednisolone sodium succinate, e.g., allergic to methylprednisolone sodium succinate, premature infant, immune system related thrombocytopathy, immunodeficiency, any congenital diseases.

Keywords: kawasaki disease aneurysm formation coronary artery dilatation methylprednisolone pulse therapy